Liver

Toxic Hepatitis

The liver processes almost everything a person consumes, including chemicals, medications and alcohol. In some instances, these substances may cause serious injury to the liver, resulting in toxic hepatitis — a condition that causes inflammation of the liver.

Some of the causes of toxic hepatitis include:

  • Medications
  • Alcohol
  • Herbs and Nutritional Supplements
    • Comfrey
    • Chaparral
    • Germander
    • Kava
    • Valerian
    • Mistletoe
    • Traditional Chinese herbs

People with liver disease should not use herbs or nutritional supplements without the approval of their doctor.

Signs and symptoms of toxic hepatitis vary, depending on the cause. However, some of the more common include:

  • Jaundice, a condition that causes a yellow tint in the skin and eyes
  • Fatigue
  • Loss of appetite
  • Nausea and vomiting
  • Headache
  • Abdominal pain
  • Dark urine
  • Diarrhea
  • White or clay-colored stools

The first and most important step in treating toxic hepatitis is to identify and eliminate the substance that is causing the problem, such as medications, herbs or alcohol.

In the case of alcohol-related liver damage, the goal of treatment is to eliminate alcohol from the diet and treat the psychological dependence on alcohol. Even at the advanced stage of cirrhosisliver disease from alcohol may show significant improvement with total abstinence from alcohol. Joining a treatment program such as Alcoholics Anonymous is highly recommended.

Urgent liver transplantation should be considered for patients with life-threatening liver damage caused by a medication, herb or nutritional supplement. Patients with end-stage cirrhosis from alcohol may be considered for transplantation. However, they are considered candidates for transplantation only if they have been completely abstinent from alcohol and in a treatment program for a minimum of six months.

Reye Syndrome

Reye syndrome is a rare but serious disease that most often affects children 6 to 12 years old. It seems to be related to the use of ASA (Aspirin) to treat some viral illnesses, such as chicken pox.

Reye syndrome primarily targets the brain and liver. Brain swelling and chemical changes in the blood from liver damage affect the entire body. Drowsiness, confusion, seizures, coma, and, in severe cases, death may result.

The cause of Reye syndrome is unknown. But the disease most often occurs in children who have recently had chicken pox (varicella) or flu (influenza) and who have also taken medicines that contain ASA. Reye syndrome is not contagious.

The most important step you can take to prevent Reye syndrome is to avoid giving ASA or products that contain ASA to anyone younger than 20 unless a doctor has specifically prescribed it.

Often symptoms of Reye syndrome appear during recovery from a viral infection, such as the flu or chicken pox, that has been treated with ASA products. Symptoms usually develop 3 to 7 days after a viral illness starts. The symptoms develop rapidly over several hours to a day or two.

The first symptoms may include:

  • Sudden onset of retching or vomiting that is not clearly due to stomach flu.
  • Strange behaviour, such as staring, irritability, personality change, and slurred speech.
  • Sluggishness, lack of energy, and loss of interest in surroundings.
  • Drowsiness that may lead to severe sleepiness.

As brain damage progresses, other symptoms may develop, including:

  • Confusion and inability to identify whereabouts or family members or to answer simple questions.
  • Aggressive behaviour, such as hitting others without reason.
  • Hyperventilation.
  • Seizures and abnormal body positioning, and coma.

If Reye syndrome is not recognized and treated promptly, death can occur.

Reye syndrome has become very rare since widespread campaigns publicized the dangers of giving ASA to young people. Because it is so rare, all other possible causes of brain and liver problems must be eliminated before Reye syndrome is diagnosed.

If your child has symptoms of Reye syndrome, get medical care immediately. Early treatment increases the chance for full recovery. If the disease is diagnosed early, most children recover from Reye syndrome in a few weeks. But some children develop permanent brain damage. The goal of treatment is to stop damage to the brain and liver and to prevent complications. All children with Reye syndrome are treated in a hospital intensive care unit.

Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic liver disease caused by progressive inflammation and scarring of the bile ducts of the liver. The inflammation impedes the flow of bile to the gut, which can ultimately lead to liver cirrhosis, liver failure and liver cancer. Autoimmunity is believed to be the underlying cause of the inflammation.

Approximately three out of every 100,000 individuals in the world are diagnosed with sclerosing cholangitis. Males are twice as likely than females to get PSC. The disease normally starts from age 20 to 30, though may begin in childhood. PSC progresses slowly, so the disease can be active for a long time before it is noticed or diagnosed.

In the early stages of primary sclerosing cholangitis (PSC), you may not experience any symptoms at all. As the disease progresses, symptoms may come and go and are caused by the bile not being drained properly. This can affect liver function and cause the bile to seep into your bloodstream.

Symptoms may include:

  • Itching
  • Chronic fatigue
  • Jaundice, yellowing of the skin and eyes
  • Loss of appetite
  • Weight loss
  • Chronic fatigue
  • Chills
  • Fever
  • Upper abdominal tenderness

Bile assists in the breakdown and absorption of fat. The absence of bile leads to fat malabsorption and deficiencies of fat-soluble vitamins (A, D, E, K).

Sclerosing cholangitis is a disease that continues to advance and tends to become more severe over time. Medication does not have a major impact on slowing the progression of primary sclerosing cholangitis, but it has an important role in treating complications.

Children with primary sclerosing cholangitis account for about 2 percent of all liver transplants done in children. Liver transplantation is successful in 90 percent of these patients, who go on to have a good quality of life after recovery.

Currently there is no cure for primary sclerosing cholangitis. Treatment is directed at managing symptoms and opening narrowed bile ducts. Symptoms of this disease can sometimes be managed by:

  • Antibiotics to treat bile ducts that have become infected
  • Diets low in salt and medication to treat swelling of the stomach and feet caused by fluid retention
  • Vitamin supplements since people with primary sclerosing cholangitis often do not have enough vitamins A, D and K
  • Medications (such as Cholestyramine and Ursodiol) to control itching caused by too much bile in the bloodstream, and to improve bile flow

Endoscopic or surgical procedures may be used to open major blockages in bile ducts. A catheter is a thin, flexible tube used to drain bile from the ducts and relieve the obstruction. A prosthesis (artificial device in the form of a hollow tube) may be placed in the bile ducts after they have been opened in order to keep the ducts open.

Liver transplantation may be an option if the liver begins to fail. Liver transplantation is very effective in the treatment of patients with advanced liver disease caused by primary sclerosing cholangitis. If a transplant is the best treatment option, the care team will focus on preventing complications and treating symptoms while awaiting a donated liver.

Primary Biliary Cirrhosis

Primary biliary cirrhosis (PBC) is an autoimmune disease of the liver characterized by the slow progressive destruction of the small bile ducts within the liver. When these ducts are damaged, bile builds up in the liver and over time damages the tissue. This can lead to scarring, fibrosis and cirrhosis.

PBC affects up to 1 in 3-4,000 people with 90% of those afflicted being female.

The initial signs and symptoms of the disease may be subtle, detectable only with blood testing. As the disease progresses over time, usually years, patients may suffer from itching and fatigue. At an advanced stage, jaundice — a condition that causes a yellowing tint to the skin and eyes — develops along with other signs of liver failure. These may include memory problems, fluid retention and internal bleeding.

There is no known cure for PBC at this time. Medication may slow the progression of the disease enough so that a normal lifespan and quality of life may be attainable for many patients. Treatment for fatigue, which may be debilitating in some patients, is currently unavailable.

  • Ursodeoxycholic acid (Ursodiol) is the most frequently used treatment. This helps reduce the cholestasis and improves blood test results (liver function tests).
  • To relieve itching caused by bile acids in circulation, which would normally be removed by the liver, a bile acid sequestrant may be prescribed. It absorbs bile acids in the gut so they can be eliminated, rather than re-enter the blood stream.
  • Patients with PBC have poor lipid-dependent absorption of Vitamins A, D, E, K so multivitamins (especially Vitamin D) and calcium are recommended.

As in all liver diseases, alcoholic beverages should be avoided.

In advanced cases, patients may need to undergo a liver transplant.

Non-Alcoholic Fatty Liver Disease

Nonalcoholic fatty liver disease (NAFLD) is a term used to describe the accumulation of fat in the liver of people who drink little or no alcohol. By definition, alcohol consumption of over 20 g/day (about 25 ml/day) excludes the condition.

Nonalcoholic fatty liver disease is common and, for most people, causes no signs or symptoms. But in some people, the fat that accumulates can cause inflammation and scarring in the liver. Non-alcoholic steatohepatitis (NASH) is the most extreme form of NAFLD, which is regarded as a major cause of cirrhosis of the liver of unknown cause. At its most severe, nonalcoholic fatty liver disease can progress to liver failure.

Indian men have a high prevalence of non-alcoholic fatty liver disease. Two genetic mutations for this susceptibility have been identified.

The signs and symptoms of nonalcoholic fatty liver disease vary widely, depending on the stage of the condition. During the early or middle stages of the disease, patients typically have no symptoms directly related to liver disease. However, as the disease advances, patients may experience symptoms of cirrhosis, including:

  • Fatigue
  • Memory loss or confusion
  • Fluid retention in the abdomen or legs
  • Passage of tarry black stools, which suggests internal bleeding

No standard treatment for nonalcoholic fatty liver disease exists. Instead, doctors typically work to treat the risk factors that contribute to your liver disease. Because NAFLD is related to insulin resistance and the metabolic syndrome, and may respond to treatments originally developed for other insulin-resistant states (e.g. diabetes mellitus type 2), such as weight loss, metformin and thiazolidinediones.

Treatments for the risk factors of NAFLD include:

  • Diet changes
  • Gradual weight loss may improve the process in obese patients
  • Weight-loss surgery leads to improvement and or resolution of NASH
  • Insulin sensitisers
  • Vitamin E

Liver Disease

The liver, the largest of the internal organs, essentially monitors all materials absorbed from the diet, converting nutrients to useable form and eliminating potential toxins. It also produces a number of substances essential for life.

Liver disease (also called hepatic disease) is a broad term describing any single number of diseases affecting the liver.

The causes of liver disease range from viruses to gene mutations to environmental factors including medications and alcohol. These diseases typically progress slowly because the organ has a large capacity and can regenerate. These conditions, however, can reach the stage of cirrhosis and in some instances, acute liver failure, which requires urgent evaluation at a transplant center.

Some of the diseases that affect the liver include:

  • Hepatitis – inflammation of the liver, caused mainly by various viruses but also by some poisons (e.g. alcohol), autoimmunity (autoimmune hepatitis) or hereditary conditions.
  • Non-alcoholic fatty liver disease – associated with obesity and characterized as an abundance of fat in the liver; may lead to a hepatitis, i.e. steatohepatitis and/or cirrhosis.
  • Cirrhosis – the formation of fibrous tissue in the liver from replacing dead liver cells. The death of the liver cells can be caused by viral hepatitis, alcoholism or contact with other liver-toxic chemicals.
  • Hemochromatosis – a hereditary disease causing the accumulation of iron in the body, eventually leading to liver damage.
  • Cancer of the liver – primary hepatocellular carcinoma or cholangiocarcinoma and metastatic cancers, usually from other parts of the gastrointestinal tract.
  • Wilson’s disease – a hereditary disease which causes the body to retain copper.
  • Primary sclerosing cholangitis – an inflammatory disease of the bile duct.
  • Primary biliary cirrhosis – autoimmune disease of small bile ducts.
  • Budd-Chiari syndrome – obstruction of the hepatic vein.
  • Gilbert’s syndrome – a genetic disorder of bilirubin metabolism.
  • Glycogen storage disease type II – the build-up of glycogen causes progressive muscle weakness (myopathy) throughout the body and affects various body tissues, particularly in the heart, skeletal muscles, liver and nervous system.

Symptoms of liver disease include:

  • Coated tongue
  • Itchy skin
  • Excessive sweating
  • Offensive body odor
  • Dark circles under the eyes
  • Red swollen and itchy eyes
  • Acne rosacea
  • Brownish spots and blemishes on the skin
  • Flushed facial appearance
  • Excessive facial blood vessels
  • Jaundice
  • Dark urine
  • Pale stool
  • Bone loss
  • Easy bleeding
  • Small, spider-like blood vessels visible in the skin
  • Enlarged spleen
  • Fluid in the abdominal cavity
  • Chills
  • Pain from the biliary tract or pancreas
  • An enlarged gallbladder

The only real treatment for chronic liver disease at present is a liver transplant.

Biliary Atresia

Biliary atresia is a rare disease of the liver and bile ducts that occurs in infants.

Cells within the liver produce liquid called bile. Bile helps to digest fat. It also carries waste products from the liver to the intestines for excretion. This network of channels and ducts is called the biliary system. When the biliary system is working the way it should, it lets the bile drain from the liver into the intestines.

When a baby has biliary atresia, bile flow from the liver to the gallbladder is blocked. This causes the bile to be trapped inside the liver, quickly causing damage and scarring of the liver cells (cirrhosis), and finally liver failure.

About one in 15,000 to 20,000 babies do not have complete bile ducts. Biliary atresia seems to affect girls more than boys. Within the same family, it is common for only one child in a pair of twins or only one child within the same family to have it. Asians and African-Americans are affected more frequently than Caucasians. There does not appear to be any link to medications taken during pregnancy.

The causes of biliary atresia are not completely understood. For some children, biliary atresia may occur because the bile ducts did not form properly during pregnancy. For other children with biliary atresia, the bile ducts may be damaged by the body’s immune system in response to a viral infection acquired after birth.

Ten to 15 percent of infants with biliary atresia may be born with other problems in the:

  • Heart
  • Spleen (polysplenia)
  • Blood vessels (inferior vena caval anomalies, pre-duodenal portal vein)
  • Intestine (situs-inversus or malrotation)

Babies with biliary atresia usually appear healthy when they are born. Symptoms of the disease typically appear or develop about two to eight weeks after birth. Those symptoms include:

  • Jaundice — a yellow coloring of the skin and eyes due to a very high level of bilirubin (bile pigment) in the bloodstream. Jaundice caused by an immature liver is common in newborns. It usually goes away within the first week to 10 days of life. A baby with biliary atresia usually appears normal at birth, but develops jaundice at two or three weeks after birth.
  • Dark urine — caused by the build-up of bilirubin (a breakdown product from hemoglobin) in the blood. The bilirubin is then filtered by the kidney and removed in the urine.
  • Acholic stools (clay-colored stools) — because no bile or bilirubin coloring is being emptied into the intestine. Also, the abdomen may become swollen from a firm, enlarged liver.
  • Weight loss and irritability — develop when the level of jaundice increases.

Jaundice may be present with other liver disorders, so several tests are needed to get the correct diagnosis.

Children with liver disease have a faster metabolism than healthy children. This means that children with biliary atresia may require more calories. A child with biliary atresia cannot properly digest fats. This is because not enough bile gets to the intestine. Due to liver damage, there may also be a loss of vitamins and protein. Guidelines from your doctor for your child’s nutrition may include:

  • A well-balanced diet, consisting of three meals a day plus small snacks in between meals.
  • Vitamin supplements.
  • Adding medium-chain triglyceride (MCT) oil to foods and liquids or infant formulas. MCT adds extra calories that will help your child grow.
  • High-calorie liquid feedings may be recommended if your child is too ill to eat normally. Feedings are given through a special tube (nasogastric tube) that is placed in the nose and guided down the esophagus and into the stomach.

Although digestion may return to normal after surgery, extra vitamins or MCT oil may be needed.

Biliary atresia cannot be treated with medication. A Kasai procedure or hepatoportoenterostomy is done. The Kasai procedure is an operation to create an open duct so bile can drain from the liver.

With an experienced surgeon, the Kasai procedure is successful in 60 to 85 percent of the patients. This means that bile drains from the liver and the jaundice goes down.

The Kasai procedure is not a cure for biliary atresia, but it does allow babies to grow and have fairly good health for several years. When this procedure does not work, it is usually because the blocked bile ducts are inside the liver (intrahepatic), as well as outside the liver (extrahepatic). If this is the case, liver transplantation can correct this problem.

Success with this procedure is related to:

  • Age. Surgery is most successful in infants younger than two to three months of age.
  • Extent of liver damage (cirrhosis) at the time of surgery.
  • The number and size of microscopic ducts in the scarred tissue that can drain bile.

Complications right after surgery are low. Most problems that develop are because the biliary atresia is getting worse.

Long-term survival after the Kasai procedure is affected by the presence of progressive liver disease (cirrhosis) and the development of portal hypertension (high blood pressure in the portal vein that carries blood to the liver). Nearly one-half of all infants who have had a Kasai procedure require liver transplantation before age 5. Older children may continue to have good bile drainage and no jaundice.

Some children may develop portal hypertension and have gastrointestinal bleeding, accumulation of fluid in the abdomen (ascites) and overactivity of the spleen (hypersplenism).

Eighty-five percent of all children who have biliary atresia will need to have a liver transplant before they are 20 years old. The remaining 15 percent have some degree of liver disease. Their disease can be managed without having a transplant.

If there is still not enough bile flow with the Kasai procedure, liver transplantation is a final option. A liver transplant operation removes the damaged liver and replaces it with a new liver from a donor.

After transplant surgery, the child’s health may improve quite quickly. However, the child’s body might reject the new organ. To prevent rejection, a strict schedule of anti-rejection medications must be taken. After a transplant, ongoing lifelong care is required. Frequent contact with physicians and other members of the transplant team is also necessary.

Autoimmune Hepatitis

Autoimmune hepatitis is a disease of the liver that occurs when a person’s immune system attacks its own liver cells. The immune response causes inflammation of the liver and the disease can lead to other symptoms, including cirrhosis—scarring and hardening—of the liver.

Autoimmune hepatitis has an incidence of 1-2 per 100,000 per year. It affects women 70% more often than men. It can occur at any age but most often starts in adolescence or young adulthood.

The disease is usually quite serious and, if not treated, gets worse over time. Autoimmune hepatitis is typically chronic, meaning it can last for years. Eventually, liver failure can result.

Symptoms of autoimmune hepatitis vary, depending on the severity. However, some of the more common include:

  • Fatigue
  • Enlarged liver
  • Jaundice, a condition that causes a yellowing tint of the skin and eyes
  • Joint pain
  • Abdominal discomfort
  • Blood vessels that appear as “spiders” on the surface of the skin
  • Nausea
  • Vomiting
  • Loss of appetite
  • Dark urine
  • Mental confusion, which may occur in advanced stages

People in advanced stages of the disease are more likely to have symptoms related to chronic liver disease, such as fluid in the abdomen (ascites) and mental confusion. Women may stop having menstrual periods.

Treatment is with glucocorticoids with or without azathioprine. Patients who do not respond to glucocorticoids and azathioprine may be given other immunosuppressives. Treatment with medication causes remission in up to 60–80% of cases, although many will eventually experience a relapse.

Liver transplantation may be required if patients do not respond to drug therapy or when patients present with sudden and intense liver failure.

Viral Hepatitis

Viral hepatitis is liver inflammation due to a viral infection. It may present in acute (recent infection, relatively rapid onset) or chronic forms.

As viral hepatitis becomes more advanced, it may cause one or more of the following symptoms:

  • Loss of appetite
  • Nausea and/or vomiting
  • Fever
  • Dark urine
  • Stomach pain
  • Fatigue
  • Yellowing of the skin and eyes, called jaundice
  • Mood change, forgetfulness
  • Black bowel movements, which indicate bleeding into the intestine

Treatment for hepatitis varies, depending on the type and severity of the disease.

The most common causes of viral hepatitis are the five unrelated hepatotropic viruses:

  • Hepatitis A – caused by hepatitis A virus (HAV) that is transmitted by the fecal-oral route often associated with ingestion of contaminated food. There is no specific therapy for acute hepatitis A infection. Therefore, prevention is the key. An effective vaccine is available and recommended for anyone with liver disease.
  • Hepatitis B – caused by hepatitis B virus (HBV) that is transmitted through blood, tattoos, sexually, or via mother to child by breast feeding. About 25% of people with chronic hepatitis B can be cured with a drug called pegylated interferon-alpha, which is taken as a weekly injection for six months. The alternative is suppression of the virus with oral medications. Suppression is recommended for patients with elevated viral levels and evidence of advancing liver disease.
  • Hepatitis C – caused by hepatitis C virus (HCV) that is transmitted through contact with blood (including through sexual contact if the two parties’ blood is mixed) and can also cross the placenta. The most effective therapy for hepatitis C is a drug combination consisting of pegylated interferon and ribavirin. Pegylated interferon is taken weekly as an injection and ribavirin is a twice daily tablet. The treatment is a form of chemotherapy and the ability to tolerate it varies widely for each person.Liver transplant may be an option for people whose hepatitis progresses to liver failure and who fail to respond to treatment or cannot tolerate treatment. Currently, almost one half of all liver transplants in North America are performed for end-stage hepatitis C.
  • Hepatitis D – caused by the hepatitis D virus (HDV) and can only propagate in the presence of the Hepatitis B virus. Transmission of HDV can occur either via simultaneous infection with HBV or via infection of an individual previously infected with HBV.
  • Hepatitis E – caused by the hepatitis E virus (HEV). HEV has a fecal-oral transmission route.

In addition to the hepatitis viruses, other viruses that can also cause hepatitis include:

  • Herpes simplex
  • Cytomegalovirus
  • Epstein-Barr virus
  • Yellow fever

Urea Cycle Disorder

A urea cycle disorder (UCD) is an inherited disease caused by the lack of an enzyme needed to break down ammonia in the body. Ammonia is a waste product that must be removed by the body.

Everyone needs protein, which is found in foods like dairy products, meat and fish. When a person eats food that contains protein, the body breaks it down into amino acids (the building blocks of protein that are used by the body for growth and tissue repair) and uses only what it needs. It changes the rest into ammonia, which must then be removed by the body.

In a healthy person, the liver supplies six enzymes to break down the ammonia into urea, which is then removed from the body in urine. This entire process is called the urea cycle and it occurs in liver cells.

The liver in a person with urea cycle disorder is missing an enzyme necessary to convert ammonia into urea. As a result, ammonia, a highly toxic substance, builds up in the bloodstream and is not removed from the body. Untreated, the high amounts of ammonia can cause brain damage, coma and eventually death.

These urea cycle disorders are named based on the initials of the missing enzyme. They are:

  • OTC – Ornithine transcarbamylase
  • ASD – Argininosuccinic acid synthetase (Citrullinemia)
  • AG – Arginase
  • ALD – Argininosuccinase acid lyase (Argininosuccinic aciduria)
  • CPS – Carbamyl phosphate synthetase
  • NAGS – N-acetylglutamate synthetase

Urea cycle disorders occur in about one in 30,000 newborns.

Most urea cycle disorders are caused by a lack of a specific enzyme in the urea cycle. Urea cycle disorders are genetic, and most appear only if a person inherits a defective gene from both parents. If both parents carry the defective gene, there is a:

  • 25% chance that their child will develop the disorder
  • 50% chance that their child will receive one defective gene from one of the parents, which means the child will not show symptoms of the disorder; the child is a “carrier”
  • 25% chance their child will receive both normal genes, one from each parent, and will be unaffected

However, this is not the case for ornithine transcarbamylase (OTC), which is the most common urea cycle disorder. It is passed to the baby through the DNA of the mother, and while girls or boys can inherit this genetic material, it is more commonly found in boys.

This disorder is often diagnosed in infancy, but some children do not show symptoms until early childhood. In infants, the symptoms will develop within the first 24 hours of life. While all of these symptoms may not be present, usually the baby will become very sleepy and irritable and will vomit often. Seizures, breathing difficulty and coma may appear later.

Symptoms in children with mild or moderate UCD, who do not show symptoms until early childhood, may include:

  • Disliking meat or other foods rich in protein
  • Vomiting, nausea
  • Mental confusion or hyperactive behavior
  • Tired often and/or difficult to awaken
  • Coma

Treatment is a lifelong process that doesn’t cure the condition, but it can effectively manage the symptoms. Frequent blood tests are done to continue to monitor ammonia levels. Doctors in the areas of pediatrics, genetics and nutrition will work together to develop the child’s treatment plan.

The child’s treatment probably will involve:

  • Low protein, high-calorie diet. Protein in the diet is lowered by avoiding protein-rich foods. Examples of foods that provide calories without loading the body with protein are fruits, vegetables and starches. However, protein is important for growth, so the protein restriction must be done with advice from a health care professional. A dietitian will plan and update a protein-restricted diet as the child grows.
  • Medications. Some children will need to take medicine to help take extra ammonia out of the body. Oral medication is given that binds to ammonia and carries it out in the urine.
  • Amino acid supplements. Depending on the type of UCD, amino acid supplements such as arginine or citrullline may be added to the diet to help give the body what it needs to make proteins that are important for growth and tissue repair, since children with urea cycle disorder can’t make arginine on their own.
  • Liver transplantation. Because the production of urea cycle enzymes takes place in the liver, a liver transplant can be an effective treatment for urea cycle disorder if advanced liver disease has developed. Your doctor will discuss the risks of transplantation with you and a decision must be made with the consideration of the pros and cons. If a transplant is the best treatment option, the doctor and the other members of the patient care team will focus on preventing complications and will treat symptoms while your child waits for the donated liver.

Stresses on the body, such as illness, fever, surgery or an accident, can cause ammonia levels to rise. Careful care must be taken at these times and extra calories will be needed to provide the stressed body with fuel.

Visits with your doctors and other specialists will be necessary to make adjustments to diet and medication. Having your child follow the diet your doctor gives is important for the child’s health, growth and development.

There currently is no cure for urea cycle disorders. Outcomes depend on the type of urea cycle disorder, how severe it is, how early it is diagnosed and how closely the treatment plan and diet is followed. Early diagnosis and treatment are required for the child to have a chance for good results. Babies who are diagnosed in the first week of life and are put on a diet right away may do well. If the child continues to carefully follow the diet, normal brain function can be reached.

If the child does not follow the diet or if the child has stress-induced symptoms, this can lead to repeated brain swelling and irreversible brain damage. Transplants have been effective in reversing the symptoms of urea cycle disorder.